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Single-Stage Midline Unifocalization Is Associated With Excellent Outcomes in Infants of All Ages
Elisabeth Martin1, Michael Ma1, Yulin Zhang1
1Department of Cardiothoracic Surgery, Lucile Packard Children's Hospital, Stanford University, Palo Alto, CA, USA.
Insights
This study on Tetralogy of Fallot found that single-stage unifocalization surgery in infants up to 12 months old yields excellent outcomes. Older infants may have fewer pulmonary artery reinterventions after repair.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Medical Research
Background:
- Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries is a complex defect.
- Historically, single-stage unifocalization was recommended at 3-6 months.
- Many patients present for treatment after 6 months of age.
Purpose of the Study:
- To evaluate surgical outcomes of single-stage unifocalization based on patient age at repair.
- To compare outcomes for infants undergoing repair between 3 and 12 months of age.
- To determine if age at repair influences the need for pulmonary artery reinterventions.
Main Methods:
- Retrospective review of 220 patients undergoing unifocalization between 3 and 12 months.
- Patients were grouped by age: 3.0-4.9, 5.0-5.9, 6.0-7.9, and 8.0-11.9 months.
- Competing risk regression analyses were used to compare outcomes.
Main Results:
- Complete single-stage repair was achieved in 79% of patients, with no significant difference across age groups.
- Early mortality was 4%.
- Older infants (8.0-11.9 months) showed significantly fewer pulmonary artery reinterventions compared to the youngest group.
Conclusions:
- Excellent surgical outcomes are achievable with single-stage unifocalization across various infant ages.
- Surgical timing should be individualized based on clinical and anatomical factors.
- Older, clinically appropriate infants may benefit from a lower risk of pulmonary artery reinterventions.
Abstract:
ObjectiveTetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries are a complex congenital heart defect. For years, our program has recommended early single-stage midline unifocalization at three to six months of age. However, many patients are referred beyond six months. Thus, we sought to evaluate surgical outcomes according to age at repair.MethodsWe performed a retrospective review of patients who underwent unifocalization from age 3 to 12 months. These patients had not undergone prior surgery at our institution or elsewhere and were also not protocoled into early surgery. Patients were divided in the following groups: 3.0 to 4.9 (n = 61), 5.0 to 5.9 (n = 56), 6.0 to 7.9 (n = 56), and 8.0 to 11.9 (n = 47) months. Competing risk regression analyses were performed.ResultsWe included 220 patients from 2001 to 2020. Baseline characteristics were not significantly different among the four groups. Overall, single-stage complete repair with bilateral unifocalization, ventricular septal defect closure, atrial septal defect closure, and right ventricular-pulmonary artery (PA) conduit placement was achieved at the index operation in 174 (79%) patients and did not differ across age groups. Early mortality was 4% (n = 9). At one year, 91% (200/220) of the entire cohort was fully septated. Comparing with group 1, group 4 was significantly less likely to undergo any PA reinterventions [hazard ratio (HR) 0.44, 95% CI 0.21-0.92, P = .028] or surgical PA reinterventions [HR 0.12, 95% CI 0.02-0.95, P = .044] following complete repair.ConclusionsGiven the excellent outcomes across all ages, surgical timing for single-stage unifocalization should be dictated by clinical and anatomic details, with potential advantage in select clinically appropriate older infants who appear to be at a lower probability of PA reinterventions following full septation.
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